 Leukemia & Lymphoma, 2014; Early Online: 1–9 © 2014 Informa UK, Ltd. ISSN: 1042-8194 print / 1029-2403 online DOI: 10.3109/10428194.2013.876634 Correspondence: Leo I. Gordon, Northwestern University Feinberg School of Medicine, 676 N. St. Clair, Suite 850, Chicago, IL 60611, USA. Tel: 312-695-4546. Fax: 312-695-6189. E-mail: l-gordon@northwestern.edu Received 26 November 2013; revised 2 December 2013; accepted 10 December 2013 REVIEW Recent advances in mantle cell lymphoma: report of the 2013 Mantle Cell Lymphoma Consortium Workshop Leo I. Gordon 1 , Steven H. Bernstein 2 , Pedro Jares 3 , Brad S. Kahl 4 , Thomas E. Witzig 5 & Martin Dreyling 6 1 Northwestern University Feinberg School of Medicine and the Robert H. Lurie Comprehensive Cancer Center, Chicago, IL, USA, 2 University of Rochester Medical Center, Rochester, NY, USA, 3 Hospital Clinic, University of Barcelona, Barcelona, Spain, 4 University of Wisconsin, Madison, WI, USA, 5 Mayo Clinic, Minnesota, MN, USA and 6 University of Munich-Grosshadern, Munich, Germany Introduction Mantle cell lymphoma (MCL) is an aggressive B-cell lym- phoma characterized by cyclin D1 overexpression and the t(11;14) translocation. Outcomes for patients with MCL remain poor compared with other non-Hodgkin lymphoma subtypes. No curative therapy has been identiied, and no standard treatment approaches have been established for initial or subsequent therapy. Recently, there have been sub- stantial advances in the understanding of MCL biology, and clinical trials have demonstrated signiicant clinical activity with new treatment approaches. A central catalyst in MCL research has been the Lym- phoma Research Foundation (LRF), which has provided MCL-speciic research grants and created the Mantle Cell Lymphoma Consortium (MCLC), a working group of more than 100 laboratory and clinical investigators from North America and Europe focusing on MCL. For the past 10 years, the LRF MCLC has convened a workshop in which MCL investigators are invited to present results of their MCL- related studies and discuss current ideas and controversies in the ield. he 10th MCLC Scientiic Workshop was held on 24–25 April 2013, in Atlanta, Georgia. he workshop included sessions on MCL biology, prognostic markers, novel poten- tial therapeutic targets, the role of stem cell transplant and recent and ongoing clinical trials. Highlights from the work- shop are summarized in this report. Proceedings State of MCL today In the opening presentation of the session, Pedro Jares, PhD (Hospital Clinic, University of Barcelona) reviewed the cur- rent state of MCL science. Dr. Jares noted that prior to the availability of genomics research, our understanding of the molecular biology of MCL was based on identiication of the primary oncogenic event – the t(11;14) translocation – leading to elevated cyclin D1, and a few secondary genetic alterations afecting cell cycle and DNA damage response pathways. More recently, genomic studies have revealed that MCL is in fact clinically and biologically heterogeneous. Some MCLs are cyclin D1 negative, although they display similar genomic alterations. hese cases display a difer- ential miRNA proiling, and an important fraction of them carry CCND2 rearrangement [1,2]. Other studies have iden- tiied biomarkers that may be signiicant in MCL, includ- ing NOTCH1 mutations [3] and in the E3 ubiquitin ligase UBR5 [4]. Recent and ongoing research is correlating these biomarkers and expression proiles with clinical features Abstract Mantle cell lymphoma (MCL) is an aggressive B-cell non-Hodgkin lymphoma characterized by overexpression of cyclin D1 resulting from the t(11;14) chromosomal translocation. MCL is biologically and clinically heterogeneous and frequently disseminates to extranodal areas. MCL remains a clinically challenging lymphoma subtype, as there is no proven curative therapy and no standard of care has been established for initial or subsequent lines of therapy. However, there have been considerable advances in the last several years in the treatment of MCL, leading to improved survival. Recent investigations into the biology of MCL, clinically relevant biomarkers, novel therapeutic targets and new treatment strategies were discussed at a recent workshop of the Lymphoma Research Foundation’s Mantle Cell Lymphoma Consortium. The presentations are summarized in this manuscript, which is intended to highlight areas of active investigation and identify topics for future research. Keywords: Lymphoma and Hodgkin disease, immunotherapy, pharmacotherapeutics, marrow and stem cell transplant clinical results Leuk Lymphoma Downloaded from informahealthcare.com by 190.39.228.70 on 05/20/14 For personal use only.